Startle epilepsy

Startle epilepsy is a rare neurologic disease characterized by frequent and spontaneous epileptic seizures (frequently with symmetrical or asymmetrical tonic features) triggered by a normal startle in response to a sudden and unexpected somatosensory (most frequently auditory) stimulus. Falls are common and can be traumatic. In most cases, the disease is associated with spastic hemi-, di-, or tetraplegia and intellectual disability.

Cataplexy

A sudden and transient episode of bilateral loss of muscle tone, often triggered by emotions.


Total: 4

                      


(per page)
PMID (PMCID)
15668049
MIXED_SAMPLE Child
The movement disorders of Coffin-Lowry syndrome.
Stephenson JB, Hoffman MC, Russell AJ, Falconer J, Beach RC, Tolmie JL, McWilliam RC, Zuberi SM.
Brain Dev. 2005;27(2):108-13.
One of our adult patients had several types of movement disorder and epilepsy that merged seamlessly: there was true cataplexy triggered by telling a joke, something close to cataplexy ('cataplexy') triggered by sound-startle, a predominantly hypertonic reaction varying from hyperekplexia to a more prolonged tonic reaction resembling startle epilepsy, and true unprovoked epileptic seizures.
15668049
MIXED_SAMPLE Child
The movement disorders of Coffin-Lowry syndrome.
Stephenson JB, Hoffman MC, Russell AJ, Falconer J, Beach RC, Tolmie JL, McWilliam RC, Zuberi SM.
Brain Dev. 2005;27(2):108-13.
One of our adult patients had several types of movement disorder and epilepsy that merged seamlessly: there was true cataplexy triggered by telling a joke, something close to cataplexy ('cataplexy') triggered by sound-startle, a predominantly hypertonic reaction varying from hyperekplexia to a more prolonged tonic reaction resembling startle epilepsy, and true unprovoked epileptic seizures.
15668049
MIXED_SAMPLE Child
The movement disorders of Coffin-Lowry syndrome.
Stephenson JB, Hoffman MC, Russell AJ, Falconer J, Beach RC, Tolmie JL, McWilliam RC, Zuberi SM.
Brain Dev. 2005;27(2):108-13.
One of our adult patients had several types of movement disorder and epilepsy that merged seamlessly: there was true cataplexy triggered by telling a joke, something close to cataplexy ('cataplexy') triggered by sound-startle, a predominantly hypertonic reaction varying from hyperekplexia to a more prolonged tonic reaction resembling startle epilepsy, and true unprovoked epileptic seizures.
6424556
MIXED_SAMPLE Child
Hyperekplexia: a syndrome of pathological startle responses.
Saenz-Lope E, Herranz-Tanarro FJ, Masdeu JC, Chacon Pena JR.
Ann Neurol. 1984;15(1):36-41.
This unusual entity differs from startle epilepsy and cataplexy.